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Bilateral exudative retinal detachment in a case of paraganglioma

Vishal Vohra, Harshika Chawla, Praveen Malik
Bilateral exudative retinal detachment due to extra-adrenal pheochromocytomas in a pediatric age group has been
rarely reported. We report a case of a 12-year-old female child with exudative retinal detachment and hypertensive
retinopathy changes secondary to hypertensive emergency due to extra-adrenal pheochromocytoma. The patient
presented with sudden painless diminution of vision in both eyes since 15 days. Fundus examination showed exudative
retinal detachment with Grade 4 hypertensive retinochoroidopathy changes. Supine blood pressure of 190/140 mm
of Hg was recorded. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a heterogeneously
enhancing right para-aortic mass likely representing extra-adrenal pheochromocytoma. Hypertensive ocular damage
represents target organ damage in humans with systemic arterial hypertension. Fundus examination is a rapid and low
cost modality for detecting previously existing and new onset vascular damage. Prompt recognition and timely referral
of such cases have prodigious implications for both ocular and general health of a person.

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DOI:
10.4103/2320-3897.183720
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Key words: Hypertensive choroidopathy, paraganglioma, serous retinal detachment

Hypertension leads to persistent pathologic elevation of blood

pressure and exaggerated total peripheral resistance that is
related to vascular lesions within the brain, heart, kidneys,
and eyes. Rise of blood pressure causes focal and generalized
retinal arteriolar constriction, presumably mediated by
autoregulation, that if prolonged results in breakdown of the
inner blood-retinal barrier, with extravasation of plasma fluid
and red blood cells. Consequently, retinal hemorrhages, cotton
wool spots, intraretinal lipid, and, sometimes, a macular star
configuration of hard exudates are often seen.[1] Although
the retinal vascular changes and optic neuropathy secondary
to hypertension are renowned, hypertensive choroidopathy
is seldom reported.[2] We report a case of hypertensive
choroidopathy with bilateral serous retinal detachments.

Case Report
A 12-year-old girl child presented to ophthalmology outpatient
department with complaint of sudden painless diminution of
vision in both eyes since 15 days. The patient gave an account
of four to five episodes of throbbing headache associated
with sweating and nausea, which used to get resolved
spontaneously. There was history of death of her father at the
age of 28 years of similar preceding symptoms. No records of
treatment of father were available.

pin hole and refraction. Pupils were sluggishly reacting.

Intraocular pressures were 10 and 12 mm of Hg in RE and
LE, respectively. Fundus examination of both eyes showed
presence of extensive disc edema (LE>RE) extending to
peripapillary region with diffuse arteriolar attenuation
and a few flame-shaped hemorrhages surrounding the disc
[Figure1]. Macular fan was seen on nasal side of the macula
in both the eyes. Elschnig spots were also seen temporal to
macular area [Figure 2]. Inferior retinal detachment with a
smooth surface, spanning an area from 3 to 9O clock just
below the lower extent of macula in prone position was
seen [Figure3]. Detachment extended to macula in supine
position due to shifting of fluid. B mode ultrasound with
A scan overlay showed inferior retinal detachment with
subretinal fluid shifting to the most dependent position
on movement of head position [Figure 4], confirming the
diagnosis of exudative retinal detachment in both eyes.
Supine blood pressure of 190/140 mm of Hg was recorded.
Patient was then immediately referred to pediatric department
for urgent management and workup of the cause of malignant

Ophthalmologic examination revealed visual acuity

of counting fingers at 2 meters in right eye (RE) and at
1meter in left eye (LE) with no improvement of vision on
Department of Ophthalmology, Dr. Ram Manohar Lohia Hospital
and Post Graduate Institute of Medical Education and Research,
New Delhi, India
Address for correspondence: Dr. Harshika Chawla, Doctor Hostel,
Room317, Department of Ophthalmology, Dr. Ram Manohar Lohia
Hospital and Post Graduate Institute of Medical Education and Research,
New Delhi - 110 001, India. E-mail: hannah.chawla@gmail.com
Manuscript received: 30.10.2014; Revision accepted: 14.04.2015

92

Figure 1: Colored fundus photographs showing extensive disc edema

with diffuse arteriolar attenuation and a few flame shaped hemorrhages
surrounding the disc and macular fan on nasal side of the macula in
both the eyes

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Brief Communications

Figure 3: Colored fundus photographs of both eyes showing inferior

retinal detachment

Figure 2: Elschnig spots seen temporal to macula

Figure 4: B scan with A scan overlay showing inferior retinal

detachment with shifting subretinal fluid

hypertension. She was started on sodium nitroprusside and

amlodipine for management of hypertensive emergency and
was asked to rest in propped up position to minimize the
extension of retinal detachment into macular region.
Routine laboratory workups were normal. Contrast-enhanced
computed tomography (CECT) of the abdomen revealed a
heterogeneously enhancing right para-aortic mass causing
right ureteric compression with right sided moderate
hydronephrosis, which in a clinical setting of hypertensive
crisis likely represented extra-adrenal pheochromocytoma
[Figure 5]. Twenty-four hour urine metanephrines were
460 g/24 h and normetanephrines were 620 g/24 h,
confirming the diagnosis.
Patient was followed-up in pediatric surgery department.
Patient underwent surgical resection of tumor after control of
hypertension. Patient was then lost to follow-up.

Discussion
Our patient showed Grade 4 hypertensive retinochoroidopathy
with exudative retinal detachment. Although a classic ocular
Journal of Clinical Ophthalmology and Research - May-Aug 2016 - Volume 4 - Issue 2

Figure 5: CECT abdomen showing heterogeneously enhancing right

para-aortic mass causing right ureteric compression with right-sided
moderate hydronephrosis. CECT = Contrast-enhanced computed
tomography

sequelae of severe hypertension, these findings are relatively

uncommon to the extent manifested in our patient. Among
the varied etiologies of exudative retinal detachment,
malignant hypertension comprises a minor cluster of
cases. [3] Hypertension causes hypertensive retinopathy,
optic neuropathy, and choroidopathy.[4] Among the three,
hypertensive choroidopathy is less known. Hypertensive
choroidopathy usually occurs later than retinal arteriolar
narrowing and arteriovenous changes.[5]
When the choroidal vessels are severely plagued by
elevated blood pressure, as in acute hypertension, fibrinoid
necrosis of choroidal arterioles occurs, with a resultant
breakdown of the outer blood-retinal barrier. Hypertensive
choroidopathy has been reported in toxemia of pregnancy,
renal disease, pheochromocytoma, and malignant
hypertension.[6] Choroid receives sympathetic innervation
and is sensitive to circulating vasoconstrictive factors like
angiotensin II, adrenaline, and antidiuretic hormone (ADH).
These factors and neural stimulation initiate constriction
of the choroid and choriocapillaris. [7] Hypertensive
choroidopathy occurs in three phases, viz., acute ischemic,
chronic occlusive, and chronic reparative phase. Throughout
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Brief Communications

the acute ischemic phase, choroidal arterioles constrict,

causing necrosis of the retinal pigment epithelium (RPE) and
choriocapillaris leading to accumulation of exudates within
the subretinal space and focal serous retinal detachment.
The chronic occlusive phase is characterized by narrowing
or occlusion of the choroidal capillaries and leak of the RPE
overlying these regions. Degenerative RPE lesions develop
over the macula and periphery, which intensify over time.
Finally, in the chronic reparative phase, the occluded vessels
recanalize, RPE heals, and the retina reattaches. The more
ordinarily described features of hypertensive choroidopathy
are Elschnig spots which are focal areas of chronic RPE
degeneration of chronic hypertension.[6] Siegrists streaks,
linear retinal pigment epithelial changes, which are the
sequelae of acute hypertensive choroidopathy, indicate a
poor prognosis.[8] Serous retinal detachment, a prominent
feature within the animal studies, is less commonly seen
in clinical settings.[9]
Malignant hypertension can be labeled as a medical
emergency. Its rare causes are pheochromocytomas and
paragangliomas. The extra-adrenal pheochromocytomas
are referred to as paragangliomas. A majority of the
extra-adrenal tumors occur intra-abdominally (85% occur
below the diaphragm), from the organ of Zuckerkandl or
along the sympathetic chain.[10] Almost all sympathetic
extra-adrenal paragangliomas and pheochromocytomas
produce, store, metabolize, and secrete catecholamines or
their metabolites.
Most pheochromocytomas represent sporadic tumors.
However, 15-30% cases have disease as part of a familial
disorder. Sporadic tumors are usually unicentric and unilateral,
while familial ones are often multicentric and bilateral.
Familial disorders associated with adrenal pheochromocytoma
are Von HippelLindau (VHL) syndrome, multiple endocrine
neoplasia type 2 (MEN2), and neurofibromatosis type 1 (NF1);
all of which have autosomal dominant inheritance. Hereditary
pheochromocytoma typically present at a younger age than
sporadic forms.[11]
Pheochromocytoma presents with high blood pressure due
to catecholamine excess in 95% of cases.[12] Clinical picture
of hypertension vary and may show either a persistent or
paroxysmal pattern.[13] In some patients, paroxysms can
occur along with sustained hypertension. On the contrary,
a small, however a vital proportion of patients with
pheochromocytoma are normotensive. Additional symptoms
seen in pheochromocytoma patients include headache,
palpitation, anxiety, and sweating.[14] It can result in angina,
myocarditis, arrhythmias, heart attack, cardiomyopathies,
postural hypotension, organ ischemia, acute renal failure,
pulmonary edema, encephalopathy, shock, and a flamboyant
range of ocular complications.[15]
While the ultimate management of pheochromocytoma
is surgical, medical treatment still remains significant in
perioperative management of blood pressure, as well as
94

in cases of inoperable metastatic disease. It is a widely

acknowledged fact that tumor handling during surgery can
unharness tremendous amounts of catecholamines into
circulation, which could overpower the pharmacological
antagonism. So as to control blood pressure before the
surgery, even in preoperative normotensive patients, it
is advocated that patients undergo preoperative medical
treatment.[16] Phenoxybenzamine is a noncompetitive alpha
antagonist that reduces blood pressure fluctuation and blocks
vasoconstriction, preventing an intraoperative hypertensive
surge.[13]

Conclusion
Hypertensive ocular damage represents target organ damage
in humans with systemic arterial hypertension. Fundus
examination is a rapid and low cost modality for detecting
previously existing and new onset vascular damage. For
prompt recognition and timely referral of such cases have
prodigious implications for both ocular and general health
of a person.

References
1. Lee AG, Beaver HA. Acute bilateral optic disk edema with
a macular star figure in a 12-year-old girl. Surv Ophthalmol
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retinopathy revisited: Some answers, more questions. Br J
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and the eye: Changing perspectives. J Hum Hypertens
2002;16:667-75.
4. Hayreh SS. Duke-elder lecture. Systemic arterial blood pressure
and the eye. Eye (Lond) 1996;10:5-28.
5. Bourke K, Patel MR, Prisant LM, Marcus DM. Hypertensive
choroidopathy. J Clin Hypertens (Greenwich) 2004;6:471-2.
6. Tso MO, Jampol LM. Pathophysiology of hypertensive retinopathy.
Ophthalmology 1982;89:1132-45.
7. Albert DM, Miller JW, Azar DT. Albert and Jakobeics principles
and practices of ophthalmology. 3rd ed. Amsterdam: Elseviers; 1663.
8. Pohl ML. Siegrists streaks in hypertensive choroidopathy. J Am
Optom Assoc 1988;59:372-6.
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choroidopathy: A case report. Int Ophthalmol 1991;15:9-14.
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review. Int J Urol 2009;6.
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Catecholamine-Secreting Tumors Three Distinct Families with
Hereditary Pheochromocytoma, Pheochromocytoma A New
View of the Old Problem, Dr. Jose Fernando Martin ed., ISBN:
978-953-307-822-9, InTech. Available from: http://www.intechopen.
com/books/pheochromocytoma-a-new-view-of-the-old-problem/
familial-catecholaminesecreting-tumors-three-distinct-familieswith-hereditary-pheochromocytoma [Last accessed on 2015 Aug].
12. Calhoun DA, Jones D, Textor S, Goff DC, Murphy TP, Toto RD,
etal. Resistant hypertension: Diagnosis, evaluation, and treatment.
A scientific statement from the American Heart Association
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Pressure Research. Hypertension 2008;51:1403-19.
13. Bravo EL, Tagle R. Pheochromocytoma: State-of-the-art and future
prospects. Endocr Rev 2003;24:539-53.
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14. M a n g e r W, G i f f o r d RW. C l i n i c a l a n d E x p e r i m e n t a l
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Cite this article as: Vohra V, Chawla H, Malik P. Bilateral exudative retinal
detachment in a case of paraganglioma. J Clin Ophthalmol Res 2016;4:92-5.
Source of Support: Nil. Conflict of Interest: None declared.

Deep anterior lamellar keratoplasty in dematiaceous keratomycosis

Shilpa Ajit Joshi, Neha Sandeep Sharma, Madan Deshpande
Deep anterior lamellar keratoplasty is a technique which has been used effectively to treat anterior stromal corneal
opacities, with the obvious benefit of preserving host endothelium. This report documents its use in a case of
dematiaceous keratomycosis resistant to conservative management. Therapeutic penetrating keratoplasty, although a
gold standard, has a high risk of rejection and poor graft survival. In our patient, careful case selection and meticulous
dissection ensured elimination of infection, restoration of vision, and absence of recurrence of infection.
Key words: Deep anterior lamellar keratoplasty, dematiaceous keratomycosis, graft survival, therapeutic penetrating
keratoplasty

Dematiaceous fungi are saprophytic pigment producing fungi

found mostly in soil and decomposing vegetative material. The
pigmented plaque infiltrate commonly seen in keratomycosis
due to this group of fungi consists of surface colonization of
pigmented fungal filaments and has been related to melanin
metabolism.
In India, the incidence of fungal keratitis is nearly
4050% of all infective keratitis[1] with dematiaceous fungal
keratitis being the 3rdmost common agent(1031%) in
fungal isolates after Fusarium and Aspergillus.[2] Commonly
reported dematiaceous fungal isolates are Curvularia and
Alternaria.
Usual treatment regime consists of topical antifungal
drops(commonly natamycin and/or voriconazole), combined
with superficial keratectomy and plaque removal. [3]
Therapeutic penetrating keratoplasty(PK) is usually
undertaken for resistant or deep keratomycosis, with
variable success rate due to high risk of rejection. [4] We
report a case of pigmented fungal keratitis with deep stromal
Department of Cornea, H. V. Desai Eye Hospital, Pune, Maharashtra,
India
Address for correspondence: Dr.Shilpa Ajit Joshi, Flat No.6, Siddh
Smruti Apartments, Plot No.35, Ashok Nagar, Range Hills Road,
Pune411007, Maharashtra, India. Email:dikeshilpa@yahoo.co.in
Manuscript received: 16.12.2014; Revision accepted: 03.02.2016

Journal of Clinical Ophthalmology and Research - May-Aug 2016 - Volume 4 - Issue 2

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DOI:
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involvement, which was managed with deep anterior lamellar

keratoplasty(DALK).

Case Report
A 50yearold female presented to us with a history of trauma
inflicted by vegetative matter to her left eye(LE), followed by
pain, redness, tearing, and progressive diminution of vision of
2weeks duration. She had been treated elsewhere with local
antibiotics. Her bestcorrected visual acuity was 6/9 in the right
eye and 2/60 in the LE, respectively on Snellen chart. The right
eye was normal but for early cataractous changes. The LE had
a diffuse conjunctival injection with circumcorneal congestion,
and a centrally located(8mm7.5mm), diffuse, elevated,
and leathery brown pigmented plaque on the corneal surface
with brownish anterior stromal infiltrates[Figure1]. There was
anterior chamber reaction of Grade1 with an immature
cataract. The posterior segment could not be visualized clearly
due to media opacity.
This is an open access article distributed under the terms of the Creative
Commons AttributionNonCommercialShareAlike 3.0 License, which allows
others to remix, tweak, and build upon the work noncommercially, as long as the
author is credited and the new creations are licensed under the identical terms.
For reprints contact: reprints@medknow.com
Cite this article as: Joshi SA, Sharma NS, Deshpande M. Deep anterior
lamellar keratoplasty in dematiaceous keratomycosis. J Clin Ophthalmol Res
2016;4:95-7.

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